Key result
Hydroxychloroquine, colchicine, and mycophenolate mofetil achieved complete pericardial effusion resolution and clinical remission over 12 months in a 16-year-old with Sjögren's syndrome.
Why the study?
Pediatric Sjogren's syndrome frequently lacks classic dryness, leading to delayed diagnosis due to atypical manifestations. Cardiac involvement, especially recurrent pericarditis, is sporadic in Sjogren's syndrome and had not been previously reported as the initial and sole presentation in pediatric patients.
Case Report (n=1)
No
This case report demonstrates that primary Sjögren's syndrome can present solely as recurrent acute pericarditis without sicca symptoms in pediatric patients, highlighting the importance of autoimmune evaluation and minor salivary gland biopsy in unexplained recurrent pericarditis.
Recurrent pericarditis may be the sole initial sign of pediatric Sjögren’s syndrome; extends the phenotype but remains hypothesis-generating.
Background: Sjögren’s syndrome (SS) is a chronic autoimmune disease primarily characterized by sicca symptoms, but its presentation in children often differs significantly from that seen in adults. In pediatric cases, classic dryness is uncommon, and the diagnosis is frequently delayed due to atypical manifestations. Cardiac involvement, especially recurrent pericarditis, is sporadic in SS and has not been previously reported as the initial and sole presentation in pediatric patients. Case Presentation: We report the case of a 16-year-old male with no prior medical history who presented with recurrent pericarditis, persistent fever, and systemic symptoms. Extensive evaluation ruled out infectious, neoplastic, and autoinflammatory causes. Although the patient lacked sicca symptoms, a minor salivary gland biopsy revealed focal lymphocytic sialadenitis with a focus score of 1.76, consistent with SS. Serologic testing was notable for isolated anti-Ro52 positivity. The patient was treated with hydroxychloroquine, colchicine, and mycophenolate mofetil, achieving clinical remission during six months of follow-up. Conclusions: This case highlights an atypical presentation of pediatric SS, manifesting solely as recurrent pericarditis without glandular involvement. It underscores the need for heightened clinical suspicion and comprehensive diagnostic evaluation in children with unexplained pericarditis, as early recognition of SS is critical to initiating appropriate immunosuppressive therapy and preventing disease progression. This case underscores the importance of considering autoimmune etiologies in recurrent pericarditis and highlights the need for increased awareness regarding early recognition of primary SS in children. Future research should focus on better defining pediatric manifestations and improving long-term follow-up strategies.
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Doğantan et al. (2026) conducted a case report in Primary Sjögren's syndrome presenting with recurrent acute pericarditis (n=1). Hydroxychloroquine, colchicine, and mycophenolate mofetil was evaluated on Clinical remission and resolution of pericardial effusion. Hydroxychloroquine, colchicine, and mycophenolate mofetil achieved complete pericardial effusion resolution and clinical remission over 12 months in a 16-year-old with Sjögren's syndrome.
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